Vasculitis
Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage. Although both occur in vasculitides, inflammation of veins or arteries on their own are separate entities.
Signs and symptoms
The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected. Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.All forms of vasculitides, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric urticaria.
| Type | Name | Main symptoms |
| Primary large vessel vasculitis | Takayasu arteritis | Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation. |
| Primary large vessel vasculitis | Giant cell arteritis | Headache, scalp tenderness, jaw claudication, and blindness. |
| Primary medium vessel vasculitis | Polyarteritis nodosa | Mononeuritis multiplex, nodules, purpura, livedo, and hypertension. |
| Primary medium vessel vasculitis | Kawasaki disease | Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema. |
| Primary small vessel antineutrophil cytoplasmic antibody –associated vasculitis | Microscopic polyangiitis | Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy. |
| Primary small vessel antineutrophil cytoplasmic antibody –associated vasculitis | Granulomatosis with polyangiitis | Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough. |
| Primary small vessel antineutrophil cytoplasmic antibody –associated vasculitis | Eosinophilic granulomatosis with polyangiitis | Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain. |
| Primary immune complex small vessel vasculitis | Anti-glomerular basement membrane disease | Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea. |
| Primary immune complex small vessel vasculitis | Cryoglobulinemic vasculitis | Palpable purpura, Raynaud's phenomenon, joint pain, and peripheral neuropathy. |
| Primary immune complex small vessel vasculitis | IgA vasculitis | Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria. |
| Primary immune complex small vessel vasculitis | Hypocomplementemic urticarial vasculitis | Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease. |
| Primary variable vessel vasculitis | Behçet's disease | Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis. |
| Primary variable vessel vasculitis | Cogan syndrome | Interstitial keratitis, ocular redness, vertigo, and tinnitus. |
| Single-organ vasculitis | Cutaneous small-vessel vasculitis | Palpable purpura, necrosis, ulceration, bullae, and nodules. |
| Single-organ vasculitis | Cutaneous arteritis | Nodules, livedo reticularis, ulcers, and gangrene. |
| Single-organ vasculitis | Primary central nervous system vasculitis | Headache, cognitive impairment, stroke, encephalopathy, and seizures. |
| Single-organ vasculitis | Retinal vasculitis | Visual impairments, floaters, and macular edema. |
| Secondary vasculitis | Lupus vasculitis | Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex. |
| Secondary vasculitis | Rheumatoid vasculitis | Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex. |
Causes
There are several different etiologies for vasculitides. Although infections usually involve vessels as a component of more extensive tissue damage, they can also directly or indirectly cause vasculitic syndromes through immune-mediated secondary events. Simple vascular thrombosis usually only affects the luminal process, but through the process of thrombus organization, it can also occasionally cause a more chronic vasculitic syndrome. The autoimmune etiologies, a particular family of diseases characterized by dysregulated immune responses that produce particular pathophysiologic signs and symptoms, are more prevalent.Classification
Primary systemic, secondary, and single-organ vasculitides are distinguished using the highest classification level in the 2012 Chapel Hill Consensus Conference nomenclature.Primary systemic vasculitis
is categorized by the size of the vessels mainly involved. Primary systemic vasculitis includes large-vessel vasculitis, medium-vessel vasculitides, small-vessel vasculitides, and variable-vessel vasculitides.Large vessel vasculitis
The 2012 Chapel Hill Consensus Conference defines large vessel vasculitis as a type of vasculitis that can affect any size artery. It usually affects the aorta and its major branches more frequently than other vasculitides. Takayasu arteritis and giant cell arteritis are the two main forms of LVV.Medium vessel vasculitis
Medium vessel vasculitis is a type of vasculitis that mostly affects the medium arteries, which are the major arteries that supply the viscera and their branches. Any size artery could be impacted, though. The two primary types are polyarteritis nodosa and Kawasaki disease.Small vessel vasculitis
Small vessel vasculitis is separated into immune complex SVV and antineutrophil cytoplasmic antibody -associated vasculitis.Antineutrophil cytoplasmic antibody -associated vasculitis is a necrotizing vasculitis linked to MPO-ANCA or PR3-ANCA that primarily affects small vessels and has few or no immune deposits. AAV is further classified as eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, and microscopic polyangiitis.
Immune complex small vessel vasculitis is a vasculitis that primarily affects small vessels and has moderate to significant immunoglobulin and complement component deposits on the vessel wall. Normocomplementemic urticarial vasculitis , cryoglobulinemic vasculitis, IgA vasculitis , and anti-glomerular basement membrane disease are the categories of immune complex SVV.
Variable vessel vasculitis
Variable vessel vasculitis is a kind of vasculitis that may impact vessels of all sizes and any type, with no particular type of vessel being predominantly affected. This category includes Behcet's disease and Cogan's syndrome.Secondary vasculitis
The subset of illnesses known as secondary vasculitides is believed to be triggered by an underlying ailment or exposure. Systemic illnesses, cancer, drug exposure, and infection are the primary causes of vasculitis; however, there are still a few factors that have a conclusively shown pathogenic relationship to the condition. Vasculitis frequently coexists with infections, and several infections, including hepatitis B and C, HIV, infective endocarditis, and tuberculosis, are significant secondary causes of vasculitis. Except for rheumatoid vasculitis, the majority of secondary vasculitis forms are exceedingly rare.Single-organ vasculitis
Single-organ vasculitis, formerly known as "localized", "limited", "isolated", or "nonsystemic" vasculitis, refers to vasculitis that is limited to one organ or organ system. Examples of this type of vasculitis include gastrointestinal, cutaneous, and peripheral nerve vasculitides.Diagnosis
- Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate, elevated C-reactive protein, anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody levels and hematuria.
- Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of organ involvement. A brain SPECT can show decreased blood flow to the brain and brain damage.
- The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
- An alternative to biopsy can be an angiogram. It can demonstrate characteristic patterns of inflammation in affected blood vessels.
- 18F-fluorodeoxyglucose positron emission tomography/computed tomography has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls. The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress.
- Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.
| Disease | Serologic test | Antigen | Associated laboratory features |
| Systemic lupus erythematosus | ANA including antibodies to dsDNA and ENA | Nuclear antigens | Leukopenia, thrombocytopenia, Coombs' test, complement activation: low serum concentrations of C3 and C4, positive immunofluorescence using Crithidia luciliae as substrate, antiphospholipid antibodies |
| Goodpasture's disease | Anti-glomerular basement membrane antibody | Epitope on noncollagen domain of type IV collagen | |
| Small vessel vasculitis | |||
| Microscopic polyangiitis | Perinuclear antineutrophil cytoplasmic antibody | Myeloperoxidase | Elevated CRP |
| Granulomatosis with polyangiitis | Cytoplasmic antineutrophil cytoplasmic antibody | Proteinase 3 | Elevated CRP |
| Eosinophilic granulomatosis with polyangiitis | perinuclear antineutrophil cytoplasmic antibody in some cases | Myeloperoxidase | Elevated CRP and eosinophilia |
| IgA vasculitis | - | ||
| Cryoglobulinemia | Cryoglobulins, rheumatoid factor, complement components, hepatitis C | ||
| Medium vessel vasculitis | |||
| Classical polyarteritis nodosa | - | Elevated CRP and eosinophilia | |
| Kawasaki's Disease | - | Elevated CRP and ESR |
In this table: ANA = antinuclear antibodies, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory